Recently published, evidence-based guidelines provide important recommendations for health maintenance, acute care, and monitoring of disease-modifying therapy in patients with sickle cell anemia (SCA). The guidelines include:

• Children with SCA should receive prophylactic penicillin from birth through at least 5 years of age, and all persons with SCA require vaccination to prevent invasive pneumococcal disease.
• Annual screening with transcranial Doppler ultrasonography is recommended for all children with SCA beginning at 2 years of age and continuing through adolescence to evaluate the risk of stroke and to initiate transfusion therapy in those at high risk.
• Vasoocclusive crises require immediate and adequate analgesia appropriate to the level of patient-reported pain.
• Antibiotics, hospitalization, and incentive spirometry are indicated for those with acute chest syndrome.
• There is strong evidence to support the promotion and use of hydroxyurea therapy in patients nine months and older who have SCA because its use can decrease the frequency of vasoocclusive crises and acute chest syndrome with limited adverse effects.